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目的:探讨特殊原因所致可逆性后部白质脑病综合征的临床和影像学的特点。方法:收集2008年3月至2014年12月年我院临床确诊的9例RPLS患者病例,回顾分析RPLS的病因、临床表现、影像特点及预后。结果:本组9例RPLS患者病因较少见,包括系统性红斑狼疮病1例、肺癌术后2例、肾移植术后1例、Miller-fisher综合征1例、Ig A肾病1例、慢性淋巴细胞性白血病1例、干燥综合征1例、肝肾综合征1例。9例患者均以急性或亚急性起病,临床表现多样。头MRI除双侧大脑后部白质对侧T1WI呈低信号,T2WI和FLAIL呈高信号改变外,双侧基底节、侧脑室旁、丘脑、脑干、小脑、胼胝体、皮质区均可受累,且DWI呈弱信号或稍高信号,ADC呈高信号改变,符合血管源性水肿的表现。结论:RPLS的神经影像学改变除典型的后部、双侧对称的白质受累病灶外,还有一些非典型的单侧白质、特殊部位及皮质受累的表现,病变具有可逆性的特点,治疗强调针对病因、控制血压、颅压、止痉、维持离子及酸碱平衡、补液、营养及支持等处理,多数病例预后良好,少数可由血管源性水肿发展为细胞毒性水肿,造成不可逆性损害。
Objective: To investigate the clinical and radiological features of reversible posterior leukoencephalopathy syndrome caused by special causes. Methods: Nine patients with RPLS diagnosed in our hospital from March 2008 to December 2014 were retrospectively analyzed. The etiology, clinical manifestations, imaging features and prognosis of RPLS were retrospectively analyzed. Results: The causes of RPLS in this group were rare, including 1 case of systemic lupus erythematosus, 2 cases of postoperative lung cancer, 1 case of renal transplantation, 1 case of Miller-fisher syndrome, 1 case of IgA nephropathy, 1 case of lymphocytic leukemia, 1 case of Sjogren’s syndrome and 1 case of hepatorenal syndrome. Nine patients were acute or subacute onset, clinical manifestations. In addition to the low signal on the contralateral white matter in T1WI and the high signal on T2WI and FLAIL in the bilateral posterior cerebral white matter, MRI showed that bilateral basal ganglia, lateral ventricle, thalamus, brainstem, cerebellum, corpus callosum and cortex could all be involved DWI was weak signal or slightly higher signal, ADC showed high signal changes, in line with the performance of vasogenic edema. CONCLUSIONS: In addition to the typical posterior and bilateral symmetrical white matter lesions, there are some atypical unilateral white matter, special parts and cortical involvement in RPLS neuroimaging. The lesions are reversible, and the treatment emphasizes For the cause, control of blood pressure, intracranial pressure, antispasmodic, maintaining ionic and acid-base balance, rehydration, nutrition and support and other treatment, the prognosis of most cases is good, a few can be vasogenic edema developed into cytotoxic edema, causing irreversible damage.